VEXAS syndrome extends the neurological complications of haemopathies

VEXAS is a complex acronym for a probably frequent disease neurologist should suggest in various neurological situations.

Bert-Marcaz et al report the first case of a CIDP (Chronic Inflammatory Demyelinating Polyneuropathy) revealing in fact a vacuole, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome. This observation involved a 74-year-old man who had developed a classical CIDP. His unsatisfactory response to Ig intravenous pulses and a high dose of steroids led clinicians to investigate further in order to determine the best second-line treatment strategy. Complementary paraclinical investigations resulted in diagnosing VEXAS, leading to targeted therapy.1

VEXAS defines a treatment refractory inflammatory syndrome, which is typically characterised by the combination of rheumatologic and haematologic features.2 The prototypical description of VEXAS syndrome would be that of an elderly man suffering from chondritis (usually nose and ear, but not the trachea), presenting both a clinical and biological inflammatory syndrome, combined with haematological…

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